Factors Influencing the Response of Infantile Epileptic Spasm Syndrome Patients to ACTH as Repeated First-Line Therapy DOI Creative Commons

Wenrong Ge,

Ping Pang,

Ziyan Zhang

et al.

Research Square (Research Square), Journal Year: 2024, Volume and Issue: unknown

Published: Oct. 11, 2024

Abstract Objective The treatment of infantile epileptic spasm syndrome (IESS) aims to achieve control. Current first-line interventions include hormonal therapy (adrenocorticotropic hormone [ACTH] and corticosteroids) vigabatrin. Despite treatment, the response rate remains at around 40%, with some patients experiencing relapse after initial In certain cases, a second course may be warranted. This study perform secondary analysis data from previously published studies by our research group elucidate factors influencing efficacy ACTH when re-administering it failure. Methods We conducted retrospective clinical children IESS who had failed or relapsed following subsequently received institution as second-line treatment. examined such variables etiological classification, interval between treatments, age first seizure, radiological findings, changes in pharmacological modalities assess their impact on short-term administration therapy. Results Among 128 identified, 50 (39.1%) achieved response. Comparative indicated that responders shorter duration since (median 1.00 [interquartile range, IQR 0.00, 2.00] vs. 1.75 [IQR 0.50, 3.88] months), were younger time repeated 11 8, 17] 16 10, 24] months, p = 0.008), less likely present additional seizure types during episodes (12.0% 28.2%, 0.030). A multivariate logistic regression model, adjusted for selected variables, revealed higher onset prior associated increased odds achieving subsequent (odds ratio [OR] 2.69, 95% confidence [CI] 1.39, 7.23, 0.014). Conversely, older onset, an etiology congenital structural anomalies without genetic aberrations lower (OR 0.85, CI 0.78, 0.92, < 0.001; OR 0.43, 0.16, 0.82, 0.032; 0.18, 0.04, 0.69, 0.016). Conclusion subset benefit trial even failure, over one-third However, alterations not suitable candidates further Additionally, correlates likelihood response, while later inversely affects probability Younger re-treatment also increase chances Consideration warranted cases

Language: Английский

Infantile epileptic spasms syndrome: an etiologic study of 361 patients with infantile epileptic spasms syndrome DOI Creative Commons

Linghui Zhu,

Yuan Xia, Hao Ding

et al.

Frontiers in Pediatrics, Journal Year: 2025, Volume and Issue: 12

Published: Jan. 9, 2025

Infantile Epileptic Spasms Syndrome (IESS) typically has a profound impact on the neurodevelopment of patients. The study IESS indicates possible geographical variation in etiology and lack data from China. Our intends to summarize analyze its characteristics. A retrospective analysis was performed gather clinical patients diagnosed with at Department Neurology Qilu Hospital Shandong University Children's Affiliated between June 2017 May 2024. total 361 were included, comprising 115 structural cases (31.9%), 37 genetic (10.2%), 32 genetic-structural (8.9%), 9 metabolic (2.5%), 3 infectious (0.8%), 165 unknown (45.7%). No immunological cause determined. primary condition linked hypoxic-ischemic encephalopathy (HIE), brain abnormalities following closely. predominant pathogenic genes identified TSC2, NF1, SCN8A, KCNQ2. Male gender, preterm infants, low birth weight developmental regression associated higher likelihood etiology. Patients exhibiting before commencement demonstrated inferior outcomes. administered adrenocorticotropic hormone (ACTH) exhibited attaining seizure control, those who responded favorably medication saw improved results. is structural, succeeded by factors, significant classifications exhibit geographic variability. Genetic etiologies are frequently an unfavorable prognosis. testing can help clarify when screening MRI results negative. advancement crucial for future targeted individualized diagnosis therapy.

Language: Английский

Citations

0
Factors Influencing the Response of Patients with Infantile Epileptic Spasms Syndrome to ACTH as Repeated First-Line Therapy DOI Creative Commons

Wenrong Ge,

Ping Pang,

Ziyan Zhang

et al.

Neurology and Therapy, Journal Year: 2025, Volume and Issue: unknown

Published: March 10, 2025

The treatment of infantile epileptic spasms syndrome (IESS) aims to achieve spasm control. Current first-line interventions include hormone therapy (adrenocorticotropic [ACTH] and corticosteroids) vigabatrin. Despite treatment, the response rate remains at around 40%, with some infants experiencing relapse after achieving initial In certain cases, a second course may be warranted. objective this study was perform secondary analysis data from our previously published studies elucidate factors influencing efficacy ACTH following its re-administration lack or relapse. We conducted retrospective clinical children IESS who had experienced failure subsequently received institution as treatment. examined such variables etiological classification, interval between treatments, age first seizure, radiological findings, changes in pharmacological modalities, overall aim assess impact these on short-term (disappearance for > 4 weeks without hypsarrhythmia pattern) administration therapy. Among 128 patients identified included analysis, 50 (39.1%) achieved response. Comparative indicated that responders shorter duration initiation (median 1.00 [interquartile range {IQR} 0.00, 2.00] vs. 1.75 [IQR 0.50, 3.88] months), were younger time 11 8, 17] 16 10, 24] months, p = 0.008), less likely present additional seizure types during episodes (12.0% 28.2%, 0.030). A multifactorial regression model older associated higher likelihood obtaining an subsequent (odds ratio [OR] 2.69, 95% confidence [CI] 1.39, 7.23, 0.014 OR 5.41, CI 1.48, 23.90, 0.016, respectively). Conversely, onset spasms, congenital structural abnormalities genetic (OR 0.85, 0.78, 0.92, < 0.001; 0.43, 0.16, 0.82, 0.032; 0.18, 0.04, 0.69, respectively).. regimen (second treatment) help did not respond relapsed, one-third responding short-term. Congenital anomalies lessen odds response, while re-treatment could improve these. reconsidered followed by

Language: Английский

Citations

0
Predictive model for initial response to first-line treatment in children with infantile epileptic spasms syndrome DOI Creative Commons

Wenrong Ge,

Lin Wan, Wang Zong

et al.

˜The œItalian Journal of Pediatrics/Italian journal of pediatrics, Journal Year: 2025, Volume and Issue: 51(1)

Published: April 12, 2025

Language: Английский

Citations

0
Factors Influencing the Response of Infantile Epileptic Spasm Syndrome Patients to ACTH as Repeated First-Line Therapy DOI Creative Commons

Wenrong Ge,

Ping Pang,

Ziyan Zhang

et al.

Research Square (Research Square), Journal Year: 2024, Volume and Issue: unknown

Published: Oct. 11, 2024

Abstract Objective The treatment of infantile epileptic spasm syndrome (IESS) aims to achieve control. Current first-line interventions include hormonal therapy (adrenocorticotropic hormone [ACTH] and corticosteroids) vigabatrin. Despite treatment, the response rate remains at around 40%, with some patients experiencing relapse after initial In certain cases, a second course may be warranted. This study perform secondary analysis data from previously published studies by our research group elucidate factors influencing efficacy ACTH when re-administering it failure. Methods We conducted retrospective clinical children IESS who had failed or relapsed following subsequently received institution as second-line treatment. examined such variables etiological classification, interval between treatments, age first seizure, radiological findings, changes in pharmacological modalities assess their impact on short-term administration therapy. Results Among 128 identified, 50 (39.1%) achieved response. Comparative indicated that responders shorter duration since (median 1.00 [interquartile range, IQR 0.00, 2.00] vs. 1.75 [IQR 0.50, 3.88] months), were younger time repeated 11 8, 17] 16 10, 24] months, p = 0.008), less likely present additional seizure types during episodes (12.0% 28.2%, 0.030). A multivariate logistic regression model, adjusted for selected variables, revealed higher onset prior associated increased odds achieving subsequent (odds ratio [OR] 2.69, 95% confidence [CI] 1.39, 7.23, 0.014). Conversely, older onset, an etiology congenital structural anomalies without genetic aberrations lower (OR 0.85, CI 0.78, 0.92, < 0.001; OR 0.43, 0.16, 0.82, 0.032; 0.18, 0.04, 0.69, 0.016). Conclusion subset benefit trial even failure, over one-third However, alterations not suitable candidates further Additionally, correlates likelihood response, while later inversely affects probability Younger re-treatment also increase chances Consideration warranted cases

Language: Английский

Citations

0